Retroperitoneal fibrosis. Vasc Med 2014 Oct;19(5):407-14
Date
08/28/2014Pubmed ID
25161213DOI
10.1177/1358863X14546160Scopus ID
2-s2.0-84910098314 (requires institutional sign-in at Scopus site) 20 CitationsAbstract
Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication. The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon.
Author List
Tzou M, Gazeley DJ, Mason PJAuthors
David J. Gazeley MD Associate Professor in the Medicine department at Medical College of WisconsinPeter Mason MD Professor in the Medicine department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
Abdominal PainArterial Occlusive Diseases
Contrast Media
Female
Follow-Up Studies
Humans
Intermittent Claudication
Middle Aged
Prednisone
Radiographic Image Enhancement
Rare Diseases
Retroperitoneal Fibrosis
Risk Assessment
Severity of Illness Index
Tomography, X-Ray Computed
Treatment Outcome
